Hypogranular variant of acute promyelocytic leukemia

Authors

  • Alejandro Llopis Giménez Resident physician. Hematology and Hemotherapy Department. Río Hortega University Hospital, Valladolid, Spain. https://orcid.org/0009-0004-5051-7419
  • Sandra Bodas del Viejo Pharmaceutical FIR. Clinical Pharmacy Service. Río Hortega University Hospital, Valladolid, Spain.
  • Monique Bourgeois Specialist Physician. Hematology Department. Río Hortega University Hospital, Valladolid, Spain.

DOI:

https://doi.org/10.24197/xf7zxb10

Keywords:

promyelocytic leukemia, PML::RARA

Abstract

Acute promyelocytic leukemia (APL) in children accounts for 5–10% of AML cases and is associated with the t(15;17) translocation and PML::RARA fusion gene.
Although considered cytogenetically favorable, it carries a high rate of early mortality due to severe hemorrhagic events.
These hemorrhages, particularly intracranial or pulmonary, are the leading cause of early death.
Diagnosis relies on cytomorphology and confirmation of PML-RARA, found in 95% of cases.
Bone marrow typically shows hypogranular and agranular blasts with irregular, monocytoid nuclei and occasional Auer rods.
Standard treatment combines all-trans retinoic acid (ATRA) and arsenic trioxide (ATO).
This regimen achieves over 85% overall survival at 5 years with relapse rates below 5%.
However, early death rates from coagulopathy have not significantly improved.
Early identification and treatment are crucial to reduce life-threatening complications.
APL remains a hematologic emergency requiring immediate intervention.

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References

Cingam SR, Koshy NV. Acute Promyelocytic Leukemia. [Updated 2023 Jun 26]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK459352/

Taga, T.; Tomizawa, D.; Takahashi, H.; Adachi, S. Acute myeloid leukemia in children: Current status and future directions. Pediatr. Int. 2016, 58, 71–80. [CrossRef] [PubMed].

Zhao J, Liang JW, Xue HL, Shen SH, Chen J, Tang YJ, Yu LS, Liang HH, Gu LJ, Tang JY, Li BS. The genetics and clinical characteristics of children morphologically diagnosed as acute promyelocytic leukemia. Leukemia. 2019 Jun;33(6):1387-1399. doi: 10.1038/s41375-018-0338-z. Epub 2018 Dec 21. PMID: 30575821.

Autora: Dra. Irene Martín de Prado

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Published

2025-10-23

Issue

No. 30 (2025): CLÍNICA

Section

Imágenes/Infografías

License

Copyright (c) 2025 Alejandro Llopis Giménez, Sandra Bodas del Viejo, Monique Bourgeois

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